Ciwon cututtukan cututtukan jini na gado

Maganin zubar jini na jini (HHT) cuta ce ta gado da jijiyoyin jini da ke haifar da zub da jini mai yawa.

HHT yana gudana ta cikin dangi a cikin tsarin ƙa'idar autosomal. Wannan yana nufin ana bukatar kwayar halittar da ba ta dace ba daga mahaifi ɗaya don a gaji cutar.

Masana kimiyya sun gano kwayoyin halittu huɗu da ke cikin wannan yanayin. Duk waɗannan kwayoyin suna da mahimmanci ga jijiyoyin jini don haɓaka yadda ya kamata. Maye gurbi a cikin ɗayan waɗannan ƙwayoyin halitta yana da alhakin HHT.

Mutanen da ke da HHT na iya haɓaka hanyoyin jini mara kyau a wurare da yawa na jiki. Waɗannan tasoshin ana kiransu malformations arteriovenous (AVMs).

Idan suna kan fata, ana kiran su telangiectasias. Shafukan da aka fi sani sun hada da lebe, harshe, kunnuwa, da yatsu. Har ila yau, jijiyoyin jini na al'ada na iya haɓaka a cikin kwakwalwa, huhu, hanta, hanji, ko wasu yankuna.

Kwayar cututtukan wannan ciwo sun hada da:

• Yawan zubar hanci a yara
• Zub da jini a cikin hanjin ciki (GI), gami da zubar jini a cikin kujerun, ko kujerun baƙi ko baƙi
• Izarfafawa ko ba a bayyana ba, ƙananan bugun jini (daga jini zuwa cikin kwakwalwa)
• Rashin numfashi
• Liverara hanta
• Ajiyar zuciya
• Anemi sakamakon ƙananan ƙarfe

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki kuma ya yi tambaya game da alamunku. Kwararren mai bada sabis na iya gano telangiectases yayin gwajin jiki. Sau da yawa akwai tarihin iyali na wannan yanayin.

Gwajin sun hada da:

• Gwajin gas din jini
• Gwajin jini
• Gwajin gwajin zuciya wanda ake kira echocardiogram
• Endoscopy, wanda ke amfani da ƙaramar kyamarar da ke haɗe da sirar bututu don duba cikin jikinku
• MRI don gano AVMs a cikin kwakwalwa
• CT ko duban dan tayi don gano AVMs a cikin hanta

Akwai gwajin kwayar halitta don neman canje-canje a cikin kwayoyin halittar da ke tattare da wannan ciwo.

Jiyya na iya haɗawa da:

• Yin tiyata don magance zubar jini a wasu yankuna
• Electrocautery (nama mai ɗumi da wutar lantarki) ko tiyata ta laser don magance yawan jini ko hanci mai ƙarfi
• Ndoarawar jijiyoyin jijiyoyin jiki (allurar wani abu ta bakin bakin bututu) don magance magudanar jini mara kyau a cikin kwakwalwa da sauran sassan jiki

Wasu mutane suna amsa maganin estrogen, wanda zai iya rage lokutan zub da jini. Hakanan za'a iya ba da baƙin ƙarfe idan akwai zubar jini mai yawa, wanda ke haifar da karancin jini. Guji shan magungunan rage jini. Wasu magungunan da ke shafar ci gaban jijiyoyin jini ana nazarin su azaman jiyya na gaba.

Wasu mutane na iya buƙatar shan maganin rigakafi kafin yin aikin hakori ko tiyata. Mutanen da ke tare da AVMs na huhu su guji yin ruwa don hana cututtukan ciki (lanƙwasa). Tambayi mai ba da sabis wasu hanyoyin kariya da ya kamata ku yi.

Waɗannan albarkatun na iya samar da ƙarin bayani game da HHT:

• Cibiyoyin Kula da Rigakafin Cututtuka - www.cdc.gov/ncbddd/hht
• HHT Cure - curehht.org
• Nationalungiyar forasa don Rare Rashin Lafiya - rarediseases.org/rare-diseases/hereditary-hemorrhagic-telangiectasia

Mutanen da ke fama da wannan ciwo na iya rayuwa daidai gwargwado, gwargwadon inda AVMs suke a cikin jiki.

Wadannan rikitarwa na iya faruwa:

• Ajiyar zuciya
• Hawan jini a huhu (hauhawar jini)
• Zuban jini na ciki
• Rashin numfashi
• Buguwa

Kirawo mai ba da sabis idan kai ko yaronka ya kasance yana yawan zubar jini ta hanci ko wasu alamun wannan cuta.

Ana ba da shawara kan dabi'un halitta don ma'aurata da suke son haihuwar yara kuma waɗanda ke da tarihin iyali na HHT. Idan kana da wannan yanayin, jiyya na likita na iya hana wasu nau'ikan shanyewar jiki da ciwon zuciya.

HHT; Ciwon Osler-Weber-Rendu; Cutar Osler-Weber-Rendu; Ciwon Rendu-Osler-Weber

• Tsarin jini
• Jijiyoyin kwakwalwa

Brandt LJ, Aroniadis OC. Magungunan jijiyoyin bugun ciki. A cikin: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger da Cututtukan Cutar hanta da na Fordtran. 10 ed. Philadelphia, PA: Elsevier Saunders; 2016: babi na 37.

Cappell MS, Lebwohl O. Magungunan zubar jini na jini a cikin: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Jiyya na cututtukan fata: Dabarun Magungunan Mahimmanci. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014: babi na 98.

McDonald J, Pyeritz RE. Ciwon cututtukan cututtukan jini na gado. A cikin: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Intanet]. Seattle, WA: Jami'ar Washington, Seattle; 1993-2019. An sabunta Fabrairu 2, 2017. An shiga Mayu 6, 2019.